Epidermolysis and Pemphigoid
Michael P. Federle, MD, FACR
Mark M. Hammer, BS, MS
Key Facts
Terminology
Epidermolysis bullosa: Rare inherited disease; causes blistering of skin and mucous membranes
Benign mucous membrane pemphigoid: Acquired autoimmune skin blistering disease
Imaging
Esophageal strictures
Most commonly in upper 1/3 of esophagus
Esophageal webs seen early in benign mucous membrane pemphigoid; occasionally seen in epidermolysis bullosa
Clinical Issues
Both diseases are rare
Epidermolysis bullosa
Presents in early childhood
Severe skin blistering, nail dystrophy
Webbing between fingers and toes, can eventually lead to contractures or amputation
Diffuse osteopenia, muscle atrophy secondary to underuse
GU abnormalities: Vaginal stenosis, bladder wall thickening
Benign mucous membrane pemphigoid
Presents in 4th decade
Bullae, preference for mucous membranes
Individual bullae may resolve or progress to ulceration or stenosisRelated posts:
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